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Clinical Case Challenges In Neuro-Optometry III

Clinical Case Challenges In Neuro-Optometry III. Thomas J. Landgraf, O.D., F.A.A.O. Before We Get Started…. On our LAST hour together Foundations & Support Groups Myasthenia Gravis Multiple Sclerosis. Case #6: Don’t Assume Anything. Or “The Case Of The Chronic Sixth Nerve Palsy”

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Clinical Case Challenges In Neuro-Optometry III

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  1. Clinical Case Challenges In Neuro-Optometry III Thomas J. Landgraf, O.D., F.A.A.O.

  2. Before We Get Started….. • On our LAST hour together • Foundations & Support Groups • Myasthenia Gravis • Multiple Sclerosis

  3. Case #6: Don’t Assume Anything • Or “The Case Of The Chronic Sixth Nerve Palsy” • 70 yo male • Diplopia with isolated abduction deficit OS • Other eye movements intact • No other significant neuro-eye findings

  4. Case #6: Don’t Assume Anything • Or “The Case Of The Chronic Sixth Nerve Palsy” • Most likely a case of ischemic sixth nerve palsy • Patient of vasculopathic age • Sixth nerve palsy is isolated • May follow the patient without neuro-imaging • Expectation of improvement or resolution in 8-12 weeks

  5. Case #6: Don’t Assume Anything • Or “The Case Of The Chronic Sixth Nerve Palsy” • No expected recovery in 6-8 weeks upon re-exam • Refer to neurologist • MRI with gadolinium • DDx includes: a mass lesion • Pons, along the clivus, in the nasopharynx, at the base of the brain, in the cavernous sinus, in the orbit

  6. Case #6: Don’t Assume Anything • Or “The Case Of The Chronic Sixth Nerve Palsy” • MRI with gadolinium • Large pontine mass consistent with glioma • A death sentence in a young person • Elderly, grows very slowly

  7. Case #6: Don’t Assume Anything • Or “The Case Of The Chronic Sixth Nerve Palsy” • Neurosurgical consult • Monitor without intervention • Prismatic correction

  8. CN VI Palsy • Background • Common cause of horizontal diplopia • Most commonly affected of the ocular motor nerves

  9. CN VI Palsy • Background • Most emergent • Age 40 and under • If non-isolated • Think microvascular in elderly • If isolated, most patients recover fully and may not require referral

  10. CN VI Palsy • Background: Anatomy • Longest subarachnoid course • Nucleus in the pons • Innervates the ipsilateral lateral rectus • T2-weighted MRI in a patient with a chronic CN VI palsy shows a left pontine glioma 

  11. CN VI Palsy • Diagnosis: Who? • Any age • Variety of causes • Ischemia most common in adults • Elderly with DM and HTN

  12. CN VI Palsy • Diagnosis: Who? • Variety of causes • Neoplasm • Mass lesion of CNS most common in children and young adults • MRI of large posterior fossa tumor associated with hydrocephalus and CN VI palsy in a 6 yo • Inflammation • Post-viral and ear infections in kids • Aneurysm • Trauma

  13. CN VI Palsy • Diagnosis: Symptoms • Diplopia • Binocular or monocular? • Horizontal or vertical? • Pain, especially if growing lesion in the cavernous sinus • Additional neurologic signs • Depends on the etiology

  14. CN VI Palsy • Diagnosis: Signs • Abduction deficit • Esotropia • Maximum on gaze to the side of the palsy • Head turn

  15. CN VI Palsy • Diagnosis: additional signs dependent on etiology • Papilledema with nausea, vomiting, tinnitus, HA’s • Proptosis • Ptosis • Increased ESR with HA and jaw claudication • Retraction of globe and narrowing of lid fissure on attempted abduction

  16. CN VI Palsy • Differential Diagnosis • Ischemia • Inflammation • Neoplasm • Aneurysm • Trauma

  17. CN VI Palsy • Ancillary Tests: Optometric In-Office • Visual fields • Forced duction?

  18. CN VI Palsy • Ancillary Tests: Referral • Indications for Neuro-Imaging • Emergent if age < 30 years • Head trauma • Pain • Non-isolated • Other etiologies besides microvascular, myasthenia gravis, thyroid, Giant Cell, congenital

  19. CN VI Palsy • Ancillary Tests: Referral • Indications for Neuro-Imaging • Consult with neuro-eye doc or neurologist reassures: • “that level of comfort thing again” • Workup if microvascular: DM, HTN

  20. CN VI Palsy • Management • Microvascular, trauma, idiopathic • Resolve spontaneously within 6 months • Comfort: patch, blur, block, Botox for temporary treatment • If need long-term: prism, surgery

  21. CN VI Palsy • Management • Follow-up • CN VI  every 6 weeks over 6 months • If you expect improvement? • Neuro consult if no improvement

  22. CN VI Palsy • My Clinical Experience • All isolated (majority) have been: • Elderly • Microvascular • If it all makes sense, I hold off on the “Neuro-massage” • All non-isolated: • Younger • Poor prognosis

  23. Case #7: A Quickie • On-call Resident • Ptosis OD • Several months prior Ptosis OS • Seen by another resident • Don’t assume you are smarter than another resident • Documentation was correct • What is really going on?

  24. Myasthenia Gravis • Background • Autoimmune Disease • Autoantibodies against acetylcholine receptors • Abnormal fatigueability of muscles under voluntary control • Usually orbital and facial muscles

  25. Myastenia Gravis • Background • Prevalence: 1:20,000 • But we see it! • Ocular involvement: 90% • Account for initial complaint in 75%

  26. Myasthenia Gravis • Diagnosis: Who? • Females under 50 / 7:3 • Males peak in late 50’s • Associated conditions: thymoma, thyroid disease, diabetes, lupus, rheumatoid

  27. Myasthenia Gravis • Diagnosis: Symptoms • Majority present with ocular symptoms • Ptosis: asymmetric • Diplopia: any motility defect • And spread • Variability of ocular fatigue • Worse at the end of the day • Hx, Hx, Hx!

  28. Myasthenia Gravis • Diagnosis: Symptoms • Non-Ocular • Within two years of ocular • Limb fatigue • Facial muscle weakness • Difficulty breathing, chewing, talking, swallowing

  29. Myasthenia Gravis • Diagnosis: Signs • Ptosis & EOM involvement • Cogan’s lid twitch • Exposure keratitis • Ophthalmoplegia • Orbicularis oculi weakness

  30. Myasthenia Gravis • Differential Diagnosis • Pupils are never affected • No eye pain • Thyroid ophthalmopathy, • INO (Internuclear Ophthalmoplegia), orbital pseudotumor, botulism, myotonic dystrophy, Chronic Progressive External Ophthalmoplegia

  31. Myasthenia Gravis • Ancillary Tests: Optometric In-Office • Measure palpebral apertures • Pupil center to upper lid margin • Sustained up gaze • Squeezing of eyelids closed • Initial VF

  32. Myasthenia Gravis • Ancillary Tests: Optometric In-Office • Ice Pack Test • 5 minutes • Improves neuromuscular transmission  ptosis • Safe, speedy, easy and with relatively high sensitivity and specificity • Sleep Test: eyes closed for 30 minutes • FAT (Family Album Topography) Scan

  33. Myasthenia Gravis • Ancillary Tests: Referral • Tensilon (Endrophonium HCL) Test • IV 10 mg of Tensilon • Why refer in Tennessee? • Rate of complications low but life-threatening • Hypotension, bradycardia, cardiac arrest, respiratory arrest, seizures, vomiting • Improves eyelid / motility defect • Anticholinesterase

  34. Myasthenia Gravis • Ancillary Tests: Referral • EMG (Electomyography) • Acetylcholine antibody receptor test

  35. Myasthenia Gravis • Management • Referral • Neurologist, neuro-eye doc • Internist or PCP • Lab testing for associated conditions • CT scan of chest / mediastinum for thymoma

  36. Myasthenia Gravis • Management: Medical • Anticholinesterases, steroids, immunosuppressants • Thymectomy • Plasmapharesis • IV gammaglobulin

  37. Myasthenia Gravis • Management: Optometric • Lid crutches, tape • Occlusion • Rarely prism, ptosis or strabismus surgery • Follow-up as needed post-diagnosis • Monitor for steroid side effects

  38. Myasthenia Gravis • My Clinical Experience • Am I missing this? • Teaching about it may help…..

  39. Case #8: OK To Not Refer? • History • 2006 68 yo Caucasian female • My patient since 1997 • “my glaucoma drops are too expensive” • Alphagan-P bid OU • H/O thinner that normal pachymetry OU • H/O highest tonometry 20 mm Hg OU

  40. Case #8: OK To Not Refer? • History: Of interest to us today….. • 1997: first visit • Referred for pupil and glaucoma work-up • Anisicoria noted over 10 years ago

  41. Case #8: OK To Not Refer? • History • HTN, hypercholesterolemia • H/O bypass • Hyzarr, Metiprolol, Lipitor, Aspirin, Lyrica, vitamins • No wonder the glc drop is too expensive • POAG, Horner’s Syndrome, ERM

  42. Case #8: OK To Not Refer? • Exam • BVA: 20/25+, 20/25+ • Pupils: anisocora • Dim illumination: 4, 6 mm • Bright illumination: 3, 4 mm • Ptosis: 1 mm upper lid OD • EOM’s: FROM • Confrontation fields: FTFC OU

  43. Case #8: OK To Not Refer? • Exam • SLX: essentially normal OU, mild NS OU • T(a): 16, 15 • DFE • .7/.7 OD, .6/.6 OS • Macular pigmentary changes • periphery clear OU • Yearly HRT, VF

  44. Case #8: OK To Not Refer • Latest VF OD

  45. Case #8: OK To Not Refer • Latest VF OS

  46. Case #8: OK To Not Refer • Latest HRT OD

  47. Case #8: OK To Not Refer • Latest HRT OS

  48. Case #8: OK To Not Refer? • Assessment • 1. POAG OU • Stable HRT, VF, ONH appearance, IOP • Alphagan-P too expensive • 2. H/O Horner’s Syndrome OD • Benign and stable • 3. Macular pigmentary changes

  49. Case #8: OK To Not Refer? • Plan: • 1. Switch Alphagan-P to Brimonidine, RTC 1 month IOP check, Education potential side effects of Brimonidine • 2,3. To monitor

  50. Case #8: OK To Not Refer? • After preparing this lecture • Looked way back in the record again • 10/5/83: “Anisocoria noted 1-2 years ago; neuro-opthalmology work-up with no known causes” • I never got the cocaine in ‘97 • Good idea eventually: • Iopidine • Paredrine or Pholedrine

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